Histopathology, Central Institute, Valais Hospital, Avenue du Grand-Champsec 86, CH-1951, Sion, Switzerland.
Unit of Pathology, Department of Medicine and Technological Innovation, University of Insubria, Varese, Italy.
Endocr Pathol. 2023 Sep;34(3):349-357. doi: 10.1007/s12022-023-09773-1. Epub 2023 May 30.
Amphicrine carcinomas are epithelial neoplasms composed of cells with co-existing exocrine-neuroendocrine phenotype and are challenging lesions from both diagnostic and therapeutic perspectives.Here, we report the case of a 63-year-old male patient with a gastric nodule that was endoscopically biopsied, revealing histological features of a type 3 well-differentiated gastric neuroendocrine tumor (NET). At imaging, the lesion was single and limited to the stomach, but did not present Octreotide uptake, despite SSTR2A immunohistochemical expression. The patient underwent a wedge resection of the gastric wall, with a final pathological diagnosis of amphicrine carcinoma with pancreatic acinar cell and neuroendocrine features (pT1b). Predictive immunohistochemistry showed microsatellite stability and negative HER2 status. Hotspot targeted deep sequencing of 57 genes showed no somatic mutation, in agreement with the low mutational burden reported for gastric amphicrine carcinomas. Due to a low stage of the tumor and the poor performance status of the patient, no additional oncological treatment was administered. The patient was disease-free after 18 months.This unusual case highlights the importance of considering amphicrine carcinoma in the diagnostic work-up of gastric type 3 NET. This can be done by including in the immunohistochemical panel non-neuroendocrine markers, such as the pancreatic acinar cell and glandular ones. Correct pathological diagnosis is pivotal to determine the appropriate staging (NET vs exocrine one) for surgical and oncological management.
交界性癌是一种上皮性肿瘤，由同时具有外分泌-神经内分泌表型的细胞组成，从诊断和治疗的角度来看，都是具有挑战性的病变。在此，我们报告了一例 63 岁男性患者的病例，其胃部有一个结节，经内镜活检显示为 3 型分化良好的胃神经内分泌肿瘤（NET）的组织学特征。在影像学上，病变为单发且局限于胃，但没有奥曲肽摄取，尽管 SSTR2A 免疫组化表达阳性。患者接受了胃壁楔形切除术，最终病理诊断为具有胰腺腺泡细胞和神经内分泌特征的交界性癌（pT1b）。预测性免疫组化显示微卫星稳定性和 HER2 状态阴性。57 个基因的热点靶向深度测序未显示体细胞突变，与报道的胃交界性癌低突变负担一致。由于肿瘤分期低且患者身体状况不佳，未给予额外的肿瘤治疗。18 个月后患者无疾病。这个不寻常的病例强调了在胃 3 型 NET 的诊断工作中考虑交界性癌的重要性。这可以通过在免疫组化面板中加入非神经内分泌标志物来实现，如胰腺腺泡细胞和腺体标志物。正确的病理诊断对于确定适当的分期（NET 与外分泌肿瘤）以进行手术和肿瘤管理至关重要。
